title: Lymphoma and cerebral vasculitis in association with X-linked lymphoproliferative disease.
abstract: Lymphoma is seen in up to 30% of patients with X-linked lymphoproliferative disease (XLP), but cerebral vasculitis related with XLP after cure of Burkitt lymphoma is rarely reported. We describe a case of a 5-year-old boy with XLP who developed cerebral vasculitis two years after cure of Burkitt lymphoma. He had Burkitt lymphoma at the age of 3 years and received chemotherapy (non-Hodgkin's lymphoma-Berlin-Frankfurt-Milan-90 protocol plus rituximab), which induced complete remission over the following two years. At the age of 5 years, the patient first developed headache, vomiting, and then intellectual and motorial retrogression. His condition was not improved after anti-infection, dehydration, or dexamethasone therapy. No tumor cells were found in his cerebrospinal fluid. Magnetic resonance imaging showed multiple non-homogeneous, hypodense masses along the bilateral cortex. Pathology after biopsy revealed hyperplasia of neurogliocytes and vessels, accompanied by lymphocyte infiltration but no tumor cell infiltration. Despite aggressive treatment, his cognition and motor functions deteriorated in response to progressive cerebral changes. The patient is presently in a vegetative state. We present this case to inform clinicians of association between lymphoma and immunodeficiency and explore an optimal treatment for lymphoma patients with compromised immune system.
authors.affiliation: State Key Laboratory of Oncology in South China; Department of Pediatric Oncology, Sun Yat-sen University Cancer Center, Guangzhou, Guangdong 510060, P. R. China. sunxf@sysucc.org.cn.
authors.forename: Jia
authors.initials: J
authors.lastname: Zhu
authors.forename: Yu
authors.initials: Y
authors.lastname: Zhang
authors.forename: Zi-Jun
authors.initials: ZJ
authors.lastname: Zhen
authors.forename: Yan
authors.initials: Y
authors.lastname: Chen
authors.forename: Juan
authors.initials: J
authors.lastname: Wang
authors.forename: Rui-Qing
authors.initials: RQ
authors.lastname: Cai
authors.forename: Xiao-Fei
authors.initials: XF
authors.lastname: Sun
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002051:Burkitt Lymphoma / Q000150:complications* / Q000188:drug therapy; D002675:Child, Preschool; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000209:etiology*; D008279:Magnetic Resonance Imaging; D008297:Male; D000069283:Rituximab; D016896:Treatment Outcome; D020293:Vasculitis, Central Nervous System / Q000209:etiology*
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab